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ACHD Guidelines Slide Set
ACC/AHA 2008 Guidelines for the Management of Adults
With Congenital Heart Disease
Developed in Collaboration With:
The American Society of Echocardiography, Heart Rhythm Society,
International Society for Adult Congenital Heart Disease, Society for
Cardiovascular Angiography and Interventions, and Society of Thoracic
Surgeons
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Special Thanks to
Slide Set Editors
Carole A. Warnes, MD, FRCP, FACC, FAHA, Co-Chair; Roberta G.
Williams, MD, MACC, FAHA, Co-Chair
The ACHD Guidelines Writing Committee Members
Thomas M. Bashore, MD, FACC
Sharon A. Hunt, MD, FACC, FAHA
John S. Child, MD, FACC, FAHA
Mary Etta King, MD, FACC, FASE§
Heidi M. Connolly, MD, FACC
Michael J. Landzberg, MD, FACC
Joseph A. Dearani, MD, FACC*
Pamela D. Miner, RN, MN, NP
Pedro del Nido, MD
Martha J. Radford, MD, FACC
James W. Fasules, MD, FACC
Edward P. Walsh, MD, FACC¶
Thomas P. Graham, Jr, MD, FACC†
Gary D. Webb, MD, FACC#
Ziyad M. Hijazi, MBBS, MPH, FACC, FSCAI‡
*Society of Thoracic Surgeons representative.
†International Society for Adult Congenital Heart Disease representative.
‡Society for Cardiovascular Angiography and Interventions representative.
§American Society of Echocardiography representative.
¶Heart Rhythm Society representative.
#Canadian Cardiovascular Society representative.
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Applying Classification of Recommendations
and Level of Evidence
Class I
Class IIa
Class IIb
Class III
Benefit >>> Risk
Benefit >> Risk
Additional studies with
focused objectives
needed
Benefit ≥ Risk
Additional studies with
broad objectives needed;
Additional registry data
would be helpful
Risk ≥ Benefit
No additional studies
needed
Procedure/ Treatment
SHOULD be
performed/
administered
IT IS REASONABLE to
perform
procedure/administer
treatment
Procedure/Treatment
MAY BE CONSIDERED
is reasonable
can be useful/effective/
beneficial
is probably recommended or
indicated
may/might be considered
may/might be reasonable
usefulness/effectiveness is
unknown /unclear/uncertain
or not well established
Procedure/Treatment
should NOT be
performed/administered
SINCE IT IS NOT HELPFUL
AND MAY BE HARMFUL
Alternative Phrasing:
should
is recommended
is indicated
is useful/effective/
beneficial
is not recommended
is not indicated
should not
is not
useful/effective/beneficial
may be harmful
3
Applying Classification of Recommendations
and Level of Evidence
Class I
Class IIa
Class IIb
Class III
Benefit >>> Risk
Benefit >> Risk
Additional studies with
focused objectives
needed
Benefit ≥ Risk
Additional studies with
broad objectives needed;
Additional registry data
would be helpful
Risk ≥ Benefit
No additional studies
needed
Procedure/ Treatment
SHOULD be
performed/
administered
IT IS REASONABLE to
perform
procedure/administer
treatment
Procedure/Treatment
MAY BE CONSIDERED
Procedure/Treatment
should NOT be
performed/administered
SINCE IT IS NOT HELPFUL
AND MAY BE HARMFUL
Level of Evidence:
Level A:
Data derived from multiple randomized clinical trials or meta-analyses
Multiple populations evaluated
Level B:
Data derived from a single randomized trial or nonrandomized studies
Limited populations evaluated
Level C:
Only consensus of experts opinion, case studies, or standard of care
Very limited populations evaluated
4
Top 10 Points to Remember from ACHD
Guidelines
1. The care of ACHD patients should be coordinated by regional ACHD
centers of excellence, providing education, patient care and follow-up
by specialists for affected individuals.
2. Individual and family psychosocial screening and information should
be included in the care of ACHD patients.
3. Thorough clinical evaluation should be undertaken before anticipated
non-cardiac surgery, including ECG, chest x-ray, TTE, and blood tests.
4. Counseling before pregnancy is important to ensure that patients
understand the risks to both mother and baby, including the risks of
transmitting coronary heart disease to their offspring. Any noncardiac surgery in cyanotic patients, however minor, is potentially lifethreatening and should be coordinated with regional centers of
excellence.
5. Pacemaker implantation can be beneficial in ACHD patients, but
access may be challenging and implantation should be performed at
centers where staff is familiar with unusual anatomy of congenital
heart defects.
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Top 10 Points to Remember on ACHD
Guidelines
6. Closure of a secundum atrial septal defect, either percutaneously or
surgically, is indicated for right atrial and right ventricular
enlargement with or without symptoms.
7. Primary imaging and hemodynamic assessment of aortic stenosis
and aortic valve disease are recommended by echocardiographyDoppler to evaluate aortic stenosis or aortic regurgitation, left
ventricular size, function and mass, and dimensions and anatomy of
the ascending aorta and associated lesions.
8. Lifelong cardiology follow-up is recommended for all patients with
aortic valve disease.
9. All patients with tetralogy of Fallot should have regular follow-up with
a cardiologist with expertise in ACHD, the frequency of which may be
determined by the extent and degree of residual abnormalities.
10. There is a lack of scientific evidence demonstrating proven benefit
for IE prophylaxis, although prophylaxis is reasonable in patients with
CHD with the highest risk for adverse outcome from infective
endocarditis.
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Recommendations for
Delivery of Care and Ensuring Access
7
Delivery of Care and Ensuring Access
The focus of current healthcare access goals for ACHD
patients should include the following:
Organization and Access
I IIa IIb III
I IIa IIb III
Strengthening organization of and access to transition
clinics for adolescents and young adults with CHD,
including funding of allied healthcare providers to provide
infrastructure comparable to that provided for children
with CHD.
Organization and Outreach
Organization of outreach and education programs for
patients, their families, and caregivers to recapture
patients leaving pediatric supervisory care or who are
lost to follow-up. Such programs can determine when and
where further intervention is required.
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Delivery of Care and Ensuring Access
The focus of current healthcare access goals for ACHD
patients should include the following:
Education
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Enhanced education of adult cardiovascular specialists
and pediatric cardiologists in the pathophysiology and
management of ACHD patients.
Regulatory Needs
A liaison with regulatory agencies at the local, regional,
state, and federal levels to create programs
commensurate with the needs of this large
cardiovascular population.
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Delivery of Care and Ensuring Access
Healthcare for ACHD patients should be coordinated by
regional ACHD centers of excellence that would serve as a
resource for the surrounding medical community, affected
individuals, and their families.
I IIa IIb III
Every academic adult cardiology/cardiac surgery center
should have access to a regional ACHD center for
consultation and referral.
I IIa IIb III
Each pediatric cardiology program should identify the
ACHD center to which the transfer of patients can be
made.
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All emergency care facilities should have an affiliation
with a regional ACHD center.
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Delivery of Care and Ensuring Access
Personnel and Services Recommended
for Regional ACHD Centers
Type of Service
•Cardiologist specializing in ACHD
•Congenital cardiac surgeon
•Nurse/physician asst./nurse practitioner
•Cardiac anesthesiologist
•Echocardiography*
•Includes TEE, intraoperative TEE
•Diagnostic catheterization*
Personnel/Resources
•One or several 24/7
•Two or several 24/7
•One or several
•Several 24/7
•Two or several 24/7
•Yes, 24/7
*These modalities must be supervised/performed and interpreted by
physicians with expertise and training in CHD.
ACHD indicates adult congenital heart disease; 24/7, availability 24 hours per
day, 7 days per week; TEE, transesophageal echocardiography
Warnes, et al. J Am Coll Cardiol 2008;52. Table 2. Published ahead of print November 7, 2008, at
http://content.onlinejacc.org/cgi/content/full/j.jacc.2008.10.001
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Delivery of Care and Ensuring Access
Personnel and Services Recommended
for Regional ACHD Centers
Type of Service
•Noncoronary interventional
catheterization
•Electrophysiology/pacing/AICD
implantation*
•Exercise testing
*These modalities must be supervised/performed and interpreted
by physicians with expertise and training in CHD.
ACHD indicates adult congenital heart disease; 24/7, availability
24 hours per day, 7 days per week; TEE, transesophageal
echocardiography; AICD, automatic implantable .
Personnel/Resources
•Yes, 24/7
•One or several
•Echocardiography
•Radionuclide
•Cardiopulmonary
•Metabolic
Warnes, et al. J Am Coll Cardiol 2008;52. Table 2. Published ahead of print November 7, 2008, at
http://content.onlinejacc.org/cgi/content/full/j.jacc.2008.10.001
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Delivery of Care and Ensuring Access
Personnel and Services Recommended
for Regional ACHD Centers
Type of Service
Personnel/Resources
•Cardiac imaging/radiology*
•Cardiac MRI
•CT scanning
•Nuclear medicine
•Multidisciplinary teams
•High-risk obstetrics
•Pulmonary hypertension
•Heart failure/transplant
•Genetics
*These modalities must be supervised/performed and interpreted by
physicians with expertise and training in CHD.
ACHD indicates adult congenital heart disease; CT, computed tomography;
MRI, magnetic resonance imaging.
Warnes, et al. J Am Coll Cardiol 2008;52. Table 2. Published ahead of print November 7, 2008, at
http://content.onlinejacc.org/cgi/content/full/j.jacc.2008.10.001
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Delivery of Care and Ensuring Access
Personnel and Services Recommended
for Regional ACHD Centers
Type of Service
•Multidisciplinary teams
Personnel/Resources
•Neurology
•Nephrology
•Cardiac pathology
•Rehabilitation services
•Social services
•Vocational services
•Financial counselors
ACHD indicates adult congenital heart disease.
Warnes, et al. J Am Coll Cardiol 2008;52. Table 2. Published ahead of print November 7, 2008, at
http://content.onlinejacc.org/cgi/content/full/j.jacc.2008.10.001
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Delivery of Care and Ensuring Access
Personnel and Services Recommended
for Regional ACHD Centers
Type of Service
•Information technology
Personnel/Resources
•Data collection
•Database support
•Quality assessment
review/protocols
ACHD indicates adult congenital heart disease.
Warnes, et al. J Am Coll Cardiol 2008;52. Table 2. Published ahead of print November 7, 2008, at
http://content.onlinejacc.org/cgi/content/full/j.jacc.2008.10.001
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Delivery of Care and Ensuring Access
Medical Passport
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I IIa IIb III
ACHD patients should carry a complete medical
“passport” that outlines specifics of their past and
current medical history, as well as contact information
for immediate access to data and counsel from local
and regional centers of excellence.
Additional Special Needs
Care of some ACHD patients is complicated by
additional special needs, including but not restricted to
intellectual incapacities or psychosocial limitations that
necessitate the inclusion of designated healthcare
guardians in all medical decision making.
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Delivery of Care and Ensuring Access
I IIa IIb III
Ensuring and Improving Communication
Every ACHD patient should have a primary care
physician. To ensure and improve communication,
current clinical records should be on file with the
primary care physician and a local cardiovascular
specialist, as well as at a regional ACHD center;
patients should also have copies of relevant records.
I IIa IIb III
Referral Relationship
Every cardiovascular family caregiver should have a
referral relationship with a regional ACHD center so
that all patients have geographically accessible care.
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Recommendations for Access to Care
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Access to Care
Specific Training and Expertise
I IIa IIb III
An individual primary caregiver or
cardiologist without specific training and
expertise in ACHD should manage the care
of adults with complex and moderate CHD
(Tables 3 and 4 in full-text guidelines) only in
collaboration with level 2 or level 3 ACHD
specialists.
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Access to Care
Types of Adult Congenital Heart Disease
of Great Complexity*
Conduits, valved or nonvalved
Pulmonary atresia (all forms)
Cyanotic congenital heart (all forms)
Pulmonary vascular obstructive disease
Double-outlet ventricle
Transposition of the great arteries
Eisenmenger syndrome
Tricuspid atresia
Fontan procedure
Truncus arteriosus/hemitruncus
Mitral atresia
Other abnormalities of atrioventricular or
ventriculoarterial connection not included
above (i.e., crisscross heart, isomerism,
heterotaxy syndromes, ventricular inversion)
Single ventricle (also called double
inlet or outlet, common, or primitive)
*These patients should be seen regularly at adult congenital heart disease centers.
Modified from Connelly et al. Canadian Consensus Conference on Adult Congenital Heart Disease 1996. Can J Cardiol 1998; 14:395-452.
Warnes, et al. J Am Coll Cardiol 2008;52. Table 3. Published ahead of print November 7, 2008, at
http://content.onlinejacc.org/cgi/content/full/j.jacc.2008.10.001
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Access to Care
Diagnoses in Adult Patients With Congenital Heart Disease of Moderate Complexity*
Diagnosis
Aorto–left ventricular fistulas
Pulmonary valve regurgitation (moderate to severe)
Anomalous pulmonary venous drainage, partial or
total
Pulmonary valve stenosis (moderate to severe)
Atrioventricular septal defects (partial or complete)
Sinus of Valsalva fistula/aneurysm
Coarctation of the aorta
Sinus venosus atrial septal defect
Ebstein’s anomaly
Subvalvar AS or SupraAS (except HOCM)
Infundibular right ventricular outflow obstruction of
significance
Tetralogy of Fallot
Ostium primum atrial septal defect
Ventricular septal defect with:
absent valve or valves, aortic regurgitation, coarctation of
the aorta, mitral disease, right ventricular outflow tract
obstruction, stradding tricuspid/mitral valve
Patent ductus arteriosus (not closed)
*These patients should be seen periodically at regional adult congenital heart disease centers. Modified with permission from Connelly et al. Canadian
Consensus Conference on Adult Congenital Heart Disease 1996. Can J Cardiol 1998;14:395–452.
AS indicates aortic stenosis; HOCM, hypertrophic obstructive cardiomyopathy; and SupraAS, supravalvular aortic stenosis.
Warnes, et al. J Am Coll Cardiol 2008;52. Table 4. Published ahead of print November 7, 2008, at
http://content.onlinejacc.org/cgi/content/full/j.jacc.2008.10.001
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Access to Care
Cardiac Follow-up – Low Risk Group
I IIa IIb III
For ACHD patients in the lowest-risk group
(simple CHD; Table 5 of the full-text
guidelines), cardiac follow-up at a regional
ACHD center is recommended at least once
to formulate future needs for follow-up.
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Access to Care
Diagnoses in Adult Patients With Simple Congenital Heart Disease*
Native disease
Repaired conditions
Isolated congenital aortic valve disease
Previously ligated or occluded ductus
arteriosus
Isolated congenital mitral valve disease
(e.g., except parachute valve, cleft
leaflet)
Repaired secundum or sinus venosus ASD
without residua
Isolated patent foramen ovale or small
atrial septal defect
Repaired ventricular septal defect without
residua
Isolated small ventricular septal defect
(no associated lesions)
Mild pulmonary stenosis
Small patent ductus arteriosus
*These patients can usually be cared for in the general medical community. Modified with permission from Connelly et al.
Canadian Consensus Conference on Adult Congenital Heart Disease 1996. Can J Cardiol. 1998;14:395–452.
Warnes, et al. J Am Coll Cardiol 2008;52. Table 5. Published ahead of print November 7, 2008, at
http://content.onlinejacc.org/cgi/content/full/j.jacc.2008.10.001
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Access to Care
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Cardiac Follow-up – Complex/Moderate CHD
Frequent follow-up (generally every 12 to 24 months) at
a regional ACHD center is recommended for the larger
group of adults with complex and moderate CHD. A
smaller group of adults with very complex CHD will
require follow-up at a regional ACHD center at a
minimum of every 6 to 12 months.
Urgent or Acute Care
Stabilized adult patients with CHD who require
admission for urgent or acute care should be
transferred to a regional ACHD center, except in some
circumstances after consultation with the patient’s
primary level 2 or level 3 ACHD specialist.
24
Access to Care
Diagnostic and Intervention Prodedures
I IIa IIb III
Diagnostic and interventional procedures including
imaging (i.e., echocardiography, magnetic resonance
imaging [MRI], or computed tomography [CT]), advanced
cardiac catheterization, and electrophysiology procedures
for adults with complex and moderate CHD should be
performed in a regional ACHD center with appropriate
experience in CHD and in a laboratory with appropriate
personnel and equipment. Personnel performing such
procedures should work as part of a team with expertise
in the surgical and transcatheter management of
patients with CHD.
25
Access to Care
I IIa IIb III
Anesthesia and Sedation
Surgical procedures that require general anesthesia or
conscious sedation in adults with moderate or complex
CHD should be performed in a regional ACHD center
with an anesthesiologist familiar with ACHD patients.
I IIa IIb III
ACHD patients should be transferred to an ACHD center
for urgent or acute care of cardiac problems.
I IIa IIb III
Adult patients with complex or high-risk CHD should be
transferred to an ACHD center for urgent or acute
noncardiac problems.
26
Access to Care
I IIa IIb III
An ACHD specialist should be notified or
consulted when a patient with simple or
low-risk CHD is admitted to a non-ACHD
center.
27
Recommendations for
Psychosocial Issues
28
Psychosocial Issues
Psychosocial Screening
I IIa IIb III
Individual and family psychosocial screening (including
knowledge assessment of cardiac disease and
management; perceptions about health and the impact
of CHD; social functioning with family, friends, and
significant others; employment and insurability status;
and screening for cognitive, mood, and psychiatric
disorders) should be part of the care of ACHD patients.
Advanced practice nurses, physician assistants,
psychologists, and social workers should play an integral
role in assessing and providing for the psychosocial
needs of ACHD patients.
29
Psychosocial Issues
Informational Tools
I IIa IIb III
Informational tools should be developed before transfer
from adolescent to adult care and used for
patient/family education regarding CHD, including
the following elements, to be provided in electronic
format:
a) Demographic data, including physician contact.
b) Description of CHD, surgeries, interventional
procedures, and most recent diagnostic studies.
c) Medications.
30
Psychosocial Issues
Health Maintenance Screening and Information
Additional health maintenance screening and information
should be offered to ACHD patients as indicated during each
visit to their ACHD healthcare provider, including the
following:
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a) Endocarditis prophylaxis measures .
I IIa IIb III
b) Exercise prescription, guidelines for exercise, and
athletic participation for patients with CHD should reflect
the published recommendations of the 36th Bethesda
Conference report.
31
Psychosocial Issues
Health Maintenance Screening and Information (cont’d)
I IIa IIb III
c) Contraception and pregnancy information, including
education regarding risk of CHD in offspring (for men
and women).
I IIa IIb III
d) General medical/dental preventive care (e.g.,
smoking cessation, weight loss/maintenance,
hypertension/lipid screening, oral care, and substance
abuse counseling).
I IIa IIb III
e) Recommended follow-up with cardiology.
32
Psychosocial Issues
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Referral and Formal Transition Process
Vocational referral and health insurance information
should be offered to ACHD patients during the transition
period and refreshed at the time of their initial
consultation in a tertiary referral center and intermittently
as indicated by their social situation.
A formal transition process should be used to provide
optimal transfer of patients into ACHD care. This process
should begin by 12 years of age and should be
individualized on the basis of the patient’s maturity level,
with the goal being to transition and ultimately transfer
the patient into adult care settings depending on the
stability of the disease and psychosocial status.
33
Psychosocial Issues
Referral and Formal Transition Process
I IIa IIb III
All ACHD patients should be encouraged to
complete an advance directive, ideally at a time
during which they are not extremely ill or
hospitalized, so that they can express their wishes
thoughtfully in a less stressful setting and
communicate these wishes to their families and
caregivers.
34
Recommendations for
Infective Endocarditis
35
Infective Endocarditis
Notification of Potential Risk for IE
I IIa IIb III
ACHD patients must be informed of their potential
risk for IE and should be provided with the AHA
information card with instructions for prophylaxis.
I IIa IIb III
When patients with ACHD present with an
unexplained febrile illness and potential IE, blood
cultures should be drawn before antibiotic treatment
is initiated to avoid delay in diagnosis due to
“culture-negative” IE.
I IIa IIb III
Transthoracic echocardiography (TTE) should be
performed when the diagnosis of native-valve IE is
suspected.
36
Infective Endocarditis
Patients with Evidence of IE
I IIa IIb III
Transesophageal echocardiography (TEE) is indicated if
TTE windows are inadequate or equivocal, in the
presence of a prosthetic valve or material or surgically
constructed shunt, in the presence of complex
congenital cardiovascular anatomy, or to define possible
complications of endocarditis (e.g., sepsis, abscess,
valvular destruction or dehiscence, embolism, or
hemodynamic instability).
I IIa IIb III
ACHD patients with evidence of IE should have early
consultation with a surgeon with experience in ACHD
because of the potential for rapid deterioration and
concern about possible infection of prosthetic material.
37
Infective Endocarditis
Dental Procedures and Patients with CHD
Antibiotic prophylaxis before dental procedures that involve
manipulation of gingival tissue or the periapical region of
teeth or perforation of the oral mucosa is reasonable in
patients with CHD with the highest risk for adverse
outcome from IE, including those with the following
indications:
I IIa IIb III
a) Prosthetic cardiac valve or prosthetic material
used for cardiac valve repair.
I IIa IIb III
I IIa IIb III
b) Previous IE.
c) Unrepaired and palliated cyanotic CHD, including
surgically constructed palliative shunts and
conduits.
38
Infective Endocarditis
Dental Procedures and Patients with CHD
I IIa IIb III
d) Completely repaired CHD with prosthetic
materials, whether placed by surgery or by catheter
intervention, during the first 6 months after the
procedure.
I IIa IIb III
e) Repaired CHD with residual defects at the site or
adjacent to the site of a prosthetic patch or
prosthetic device that inhibit endothelialization.
39
Infective Endocarditis
Antibiotic Prophylaxis and Vaginal Delivery
It is reasonable to consider antibiotic prophylaxis against
IE before vaginal delivery at the time of membrane
rupture in select patients with the highest risk of adverse
outcomes. This includes patients with the following
indications:
I IIa IIb III
a) Prosthetic cardiac valve or prosthetic material
used for cardiac valve repair.
I IIa IIb III
b) Unrepaired and palliated cyanotic CHD, and
including surgically constructed palliative shunts
conduits.
40
Infective Endocarditis
Antibiotic Prophylaxis and Vaginal Delivery
I IIa IIb III
Prophylaxis against IE is not recommended for
non dental procedures (such as
esophagogastroduodenoscopy or
colonoscopy) in the absence of active
infection.
41
Infective Endocarditis
Cardiac conditions associated with the highest risk of adverse
outcome from endocarditis for which prophylaxis with dental
procedures is reasonable
Condition
Congenital Specific Condition*
Previous infective endocarditis
Unrepaired cyanotic CHD, including palliative
shunts and conduits
Prosthetic cardiac valve or prosthetic
material used for cardiac valve repair
Completely repaired congenital heart defect
with prosthetic material or device, whether
placed by surgery or by catheter intervention,
during the first 6 months after the procedure†
*Except for the conditions listed above, antibiotic prophylaxis is
Repaired CHD with residual defects at the site
no longer recommended for any other form of CHD.
†Prophylaxis is reasonable because endothelialization of
or adjacent to the site of a prosthetic patch or
prosthetic material occurs within 6 months after the procedure.
prosthetic device that inhibit endothelialization
Modified with permission to include footnotes from Wilson et al.
Prevention of infective endocarditis: guidelines from the
American Heart Association: a guideline from the American
Heart Association Rheumatic Fever, Endocarditis, and
Cardiac transplant recipients who develop
Kawasaki Disease Committee, Council on Cardiovascular
cardiac valvulopathy
Disease in the Young, and the Council on Clinical Cardiology,
Council on Cardiovascular Surgery and Anesthesia, and the
Quality of Care and Outcomes Research Interdisciplinary
Working Group. Circulation. 2007;116:1736-54. CHD indicates
congenital heart disease.
Warnes, et al. J Am Coll Cardiol 2008;52. Table 6. Published ahead of print November 7, 2008, at
42
http://content.onlinejacc.org/cgi/content/full/j.jacc.2008.10.001
Infective Endocarditis
Congenital Cardiac Lesions and Preoperative Risk for Noncardiac Surgery
High risk
Pulmonary hypertension, primary or secondary
Cyanotic congenital heart disease New York Heart
Association class III or IV
Severe systemic ventricular dysfunction (ejection
fraction less than 35%)
Severe left-sided heart obstructive lesions
Moderate risk
Prosthetic valve or conduit
Intracardiac shunt
Moderate left-sided heart obstruction
Moderate systemic ventricular dysfunction
Warnes, et al. J Am Coll Cardiol 2008;52. Table 7. Published ahead of print November 7, 2008, at
http://content.onlinejacc.org/cgi/content/full/j.jacc.2008.10.001
43
Recommendations for
Noncardiac Surgery
44
Noncardiac Surgery
Basic Preoperative Assessment
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I IIa IIb III
Basic preoperative assessment for ACHD patients should
include systemic arterial oximetry, an ECG, chest x-ray,
TTE, and blood tests for full blood count and coagulation
screen.
It is recommended that when possible, the preoperative
evaluation and surgery for ACHD patients be performed
in a regional center specializing in congenital cardiology,
with experienced surgeons and cardiac
anesthesiologists.
45
Noncardiac Surgery
High Risk Populations and ACHD Patients
I IIa IIb III
Certain high-risk patient populations should be
managed at centers for the care of ACHD patients under
all circumstances, unless the operative intervention is
an absolute emergency. High-risk categories include
patients with the following:
a) Prior Fontan procedure.
b) Severe pulmonary arterial hypertension (PAH).
c) Cyanotic CHD.
d) Complex CHD with residua such as heart failure,
valve disease, or the need for anticoagulation.
e) Patients with CHD and malignant arrhythmias.
46
Noncardiac Surgery
Consultation and Assessment of Risk
I IIa IIb III
I IIa IIb III
Consultation with ACHD experts
regarding the assessment of risk is
recommended for patients with CHD who
will undergo noncardiac surgery.
Consultation with a cardiac
anesthesiologist is recommended for
moderate- and high-risk patients.
47
Recommendations for
Pregnancy and Contraception
48
Pregnancy and Contraception
I IIa IIb III
Consultation and Assessment of Risk
Patients with CHD should have consultation with an
ACHD expert before they plan to become pregnant to
develop a plan for management of labor and the
postpartum period that includes consideration of the
appropriate response to potential complications. This
care plan should be made available to all providers.
I IIa IIb III
Patients with intracardiac right-to-left shunting should
have fastidious care taken of intravenous lines to
avoid paradoxical air embolus.
49
Pregnancy and Contraception
Consultation and Assessment of Risk
I IIa IIb III
Prepregnancy counseling is recommended
for women receiving chronic
anticoagulation with warfarin to enable
them to make an informed decision about
maternal and fetal risks.
50
Pregnancy and Contraception
I IIa IIb III
Full Anticoagulation
Meticulous prophylaxis for deep venous thrombosis,
including early ambulation and compression
stockings, can be useful for all patients with
intracardiac right-to-left shunt. Subcutaneous heparin
or low-molecular-weight heparin is reasonable for
prolonged bed rest. Full anticoagulation can be useful
for the high-risk patient.
I IIa IIb III
Estrogen and Oral Contraceptives
The estrogen-containing oral contraceptive pill is not
recommended for ACHD patients at risk of
thromboembolism, such as those with cyanosis
related to an intracardiac shunt, severe PAH, or
Fontan repair.
51
Recommendations for Arrhythmia
Diagnosis and Management
52
Arrhythmia Diagnosis and Management
Noninvasive Testing
I IIa IIb III
I IIa IIb III
Complete and appropriate noninvasive testing, as
well as clear knowledge of the specific anatomy and
review of all surgical and procedural records, is
recommended before electrophysiological testing or
device placement is attempted in ACHD patients.
Tachycardia Management
Decisions regarding tachycardia management in
ACHD patients should take into account the broad
cardiovascular picture, particularly repairable
hemodynamic issues that might favor a surgical or
catheter-based approach to treatment.
53
Arrhythmia Diagnosis and Management
I IIa IIb III
I IIa IIb III
I IIa IIb III
Catheter Ablation Procedures
Catheter ablation procedures for ACHD patients should be
performed at centers where the staff is experienced with
the complex anatomy and distinctive arrhythmia
substrates encountered in congenital heart defects.
Pacemaker and Device Lead Placement
Pacemaker and device lead placement (or replacement)
in ACHD patients should be performed at centers where
the staff is familiar with the unusual anatomy of
congenital heart defects and their surgical repair.
Epicardial pacemaker and device lead placement should
be performed in all cyanotic patients with intracardiac
shunts who require devices.
54
Arrhythmia Diagnosis and Management
I IIa IIb III
Implantable Cardioverter Defibrillator
It is reasonable to recommend the use of an implantable
cardioverter defibrillator for any patient who has had a
cardiac arrest or experienced an episode of
hemodynamically significant or sustained ventricular
tachycardia (VT).
I IIa IIb III
Implantation of Cardiac Pacemakers
Pacemaker implantation can be beneficial in ACHD
patients with bradyarrhythmias and may be helpful in
overdrive pacing in patients with difficult-to-control
tachyarrhythmias. See ACC/AHA/Heart Rhythm Society
2008 Guidelines for Device-Based Therapy of Cardiac
Rhythm Abnormalities.
55
Arrhythmia Diagnosis and Management
Asymptomatic Adult Patients
I IIa IIb III
Pacemaker implantation may be beneficial
for asymptomatic adult patients with
resting heart rates of less than 40 beats
per minute or abrupt pauses in excess of 3
seconds.
56
Cyanotic Congential Heart Disease:
Recommendations for
Hematologic Problems
57
Hematologic Problems
I IIa IIb III
I IIa IIb III
Therapeutic Phlebotomy
Indications for therapeutic phlebotomy are
hemoglobin greater than 20 g per dL and
hematocrit greater than 65%, associated with
headache, increasing fatigue, or other symptoms of
hyperviscosity in the absence of dehydration or
anemia.
Repeated routine phlebotomies are not
recommended because of the risk of iron
depletion, decreased oxygen-carrying capacity,
and stroke.
58
Recommendations for
General Health Issues
for Cyanotic Patients
59
General Health Issues for Cyanotic Patients
Cyanotic Patients and Long Distance Flights
I IIa IIb III
I IIa IIb III
Cyanotic patients should drink
nonalcoholic and noncaffeinated fluids
frequently on long-distance flights to avoid
dehydration.
Supplemental oxygenation may be
considered for cyanotic patients during
long-distance flights.
60
Recommendations for
Interventional and Surgical
Therapy
61
Atrial Septal Defect Closures
I IIa IIb III
I IIa IIb III
I IIa IIb III
Closure of an ASD either percutaneously or
surgically is indicated for right atrial and RV
enlargement with or without symptoms.
A sinus venosus, coronary sinus, or primum
ASD should be repaired surgically rather than
by percutaneous closure.
Surgeons with training and expertise in CHD
should perform operations for various ASD
closures.
62
Atrial Septal Defect Closures
I IIa IIb III
I IIa IIb III
Surgical closure of secundum ASD is reasonable
when concomitant surgical repair/replacement of
a tricuspid valve is considered or when the
anatomy of the defect precludes the use of a
percutaneous device.
Closure of an ASD, either percutaneously or
surgically, is reasonable in the presence of:
a) Paradoxical embolism.
I IIa IIb III
b) Documented orthodeoxia-platypnea.
63
Atrial Septal Defect Closures
I IIa IIb III
Closure of an ASD, either percutaneously or surgically,
may be considered in the presence of net left-to-right
shunting, pulmonary artery pressure less than two
thirds systemic levels, PVR less than two thirds
systemic vascular resistance, or when responsive to
either pulmonary vasodilator therapy or test occlusion
of the defect (patients should be treated in conjunction
with providers who have expertise in the management
of pulmonary hypertensive syndromes).
I IIa IIb III
Concomitant Maze procedure may be considered for
intermittent or chronic atrial tachyarrhythmias in adults
with ASDs.
64
Atrial Septal Defect Closures
I IIa IIb III
Patients with severe irreversible PAH and
no evidence of a left-to-right shunt should
not undergo ASD closure.
65
Recommendations for
Surgical Ventricular
Septal Defect Closure
66
Surgical Ventricular Septal Defect Closure
I IIa IIb III
VSD Closure Operations
Surgeons with training and expertise in CHD
should perform VSD closure operations.
I IIa IIb III
Closure of a VSD is indicated when there is a
Qp/Qs (pulmonary-to-systemic blood flow ratio) of
2.0 or more and clinical evidence of LV volume
overload.
I IIa IIb III
Closure of a VSD is indicated when the patient has
a history of IE.
67
Surgical Ventricular Septal Defect Closure
VSD Closure Operations
I IIa IIb III
Closure of a VSD is reasonable when net left-to-right
shunting is present at a Qp/Qs greater than 1.5 with
pulmonary artery pressure less than two thirds of systemic
pressure and pulse volume recording (PVR) less than two
thirds of systemic vascular resistance.
I IIa IIb III
Closure of a VSD is reasonable when net left-to-right
shunting is present at a Qp/Qs greater than 1.5 in the
presence of LV systolic or diastolic failure.
I IIa IIb III
VSD closure is not recommended in patients with severe
irreversible PAH.
68
Interventional Catheterization for VSD
I IIa IIb III
Device closure of a muscular VSD may be
considered, especially if the VSD is
remote from the tricuspid valve and the
aorta, if the VSD is associated with severe
left-sided heart chamber enlargement, or
if there is PAH.
69
Left Ventricular Outflow Tract Obstructed
(LVOT) and Associated Lesions
70
Aortic Valve Repair/Replacement
and Aortic Root Replacement
Aortic Valve Repair and Replacement
I IIa IIb III
I IIa IIb III
Aortic valvuloplasty, AVR, or Ross repair is indicated
in patients with severe AS or chronic severe AR while
they undergo coronary artery bypass grafting,
surgery on the aorta, or surgery on other heart
valves.
AVR is indicated for patients with severe AS and LV
dysfunction (LV ejection fraction less than 50%).
71
Aortic Valve Repair/Replacement
and Aortic Root Replacement
Aortic Valve Repair and Replacement
I IIa IIb III
AVR is indicated in adolescents or young adults with
severe AR who have:
a) Development of symptoms.
b) Development of persistent LV dysfunction (LV
ejection fraction less than 50%) or progressive
LV dilatation (LV end-diastolic diameter standard
deviations above normal).
72
Aortic Valve Repair/Replacement
and Aortic Root Replacement
Aortic Valve Repair and Replacement
I IIa IIb III
Surgery to repair or replace the ascending aorta in a
patient with a bicuspid aortic valve (BAV) is recommended
when the ascending aorta diameter is 5.0 cm or more or
when there is progressive dilatation at a rate greater than
or equal to 5 mm per year.
I IIa IIb III
AVR is reasonable for asymptomatic patients with severe
AR and normal systolic function (ejection fraction greater
than 50%) but with severe LV dilatation (LV end-diastolic
diameter greater than 75 mm or end-systolic dimension
greater than 55 mm*).
*Consider lower threshold values for patients of small stature of either gender.
73
Aortic Valve Repair/Replacement
and Aortic Root Replacement
Aortic Valve Repair and Replacement
I IIa IIb III
Surgical aortic valve repair or replacement is
reasonable in patients with moderate AS
undergoing coronary artery bypass grafting or other
cardiac or aortic root surgery.
74
Aortic Valve Repair/Replacement
and Aortic Root Replacement
Aortic Valve Repair and Replacement
I IIa IIb III
AVR may be considered for asymptomatic patients with
any of the following indications:
a) Severe AS and abnormal response to exercise.
b) Evidence of rapid progression of AS or AR.
c) Mild AS while undergoing coronary artery bypass
grafting or other cardiac surgery and evidence of
a calcific aortic valve.
d) Extremely severe AS (aortic valve area less than
0.6 cm and/or mean Doppler systolic AV
gradient greater than 60 mm Hg) in an
otherwise good operative candidate.
75
(continues)
Aortic Valve Repair/Replacement
and Aortic Root Replacement
I IIa IIb III
Aortic Valve Repair and Replacement
e) Moderate AR undergoing coronary artery bypass
grafting or other cardiac surgery.
f) Severe AR with rapidly progressive LV dilation
when the degree of LV dilation exceeds an enddiastolic dimension of 70 mm or end-systolic
dimension of 50 mm, with declining exercise
tolerance, or with abnormal hemodynamic
responses to exercise.
76
Aortic Valve Repair/Replacement
and Aortic Root Replacement
Aortic Valve Repair and Replacement
I IIa IIb III
I IIa IIb III
Surgical Repair
Surgical repair may be considered in adults with AS or AR
and concomitant ascending aortic dilatation (ascending
aorta diameter greater than 4.5 cm) coexisting with AS or
AR.
Early surgical repair may be considered in adults with the
following indications:
a) AS and a progressive increase in ascending aortic size.
b) Mild AR if valve-sparing aortic root replacement is
being considered.
77
Aortic Valve Repair/Replacement
and Aortic Root Replacement
AVR and Asymptomatic Adults With AS
I IIa IIb III
I IIa IIb III
AVR is not useful for prevention of sudden
death in asymptomatic adults with AS who
have none of the findings listed under the
Class IIa/IIb indications.
AVR is not indicated in asymptomatic
patients with AR who have normal LV size
and function.
78
Surgical Intervention for SubAS
I IIa IIb III
Surgical Intervention and Surgical Resection
Surgical intervention is recommended for patients with
SubAS and a peak instantaneous gradient of 50 mm Hg
or a mean gradient of 30 mm Hg on echocardiographyDoppler.
I IIa IIb III
I IIa IIb III
Surgical intervention is recommended for SubAS with less
than a 50-mm Hg peak or less than a 30-mm Hg mean
gradient and progressive AR and an LV dimension at endsystolic diameter of 50 mm or more or LV ejection fraction
less than 55%.
Surgical resection may be considered in patients with a
mean gradient of 30 mm Hg, but careful follow-up is
required to detect progression of stenosis or AR.
79
Surgical Intervention for SubAS
I IIa IIb III
I IIa IIb III
Surgical Resection and Intervention
Surgical resection may be considered for patients with less
than a 50-mm Hg peak gradient or less than a 30-mm Hg
mean gradient in the following situations:
a) When LV hypertrophy is present.
b) When pregnancy is being planned.
c) When the patient plans to engage in
strenuous/competitive sports.
Surgical intervention is not recommended to prevent AR
for patients with SubAS if the patient has trivial LVOT
obstruction or trivial to mild AR.
80
Interventional and Surgical Therapy for
Supravalvular LVOT
Operative Intervention and Surgical Repair
I IIa IIb III
Operative intervention should be performed for
patients with supravalvular LVOT obstruction
(discrete or diffuse) with symptoms (i.e., angina,
dyspnea, or syncope) and/or mean gradient greater
than 50 mm Hg or peak instantaneous gradient by
Doppler echocardiography greater than 70 mm Hg.
I IIa IIb III
Interventions for coronary artery obstruction in
patients with SupraAS should be performed in ACHD
centers with demonstrated expertise in the
interventional management of such patients.
81
Interventional and Surgical Therapy for
Supravalvular LVOT
Operative Intervention and Surgical Repair
Surgical repair is recommended for adults with
lesser degrees of supravalvular LVOT
obstruction and the following indications:
I IIa IIb III
a) Symptoms (i.e., angina, dyspnea, or syncope).
b) LV hypertrophy.
I IIa IIb III
c) Desire for greater degrees of exercise or a
planned pregnancy.
d) LV systolic dysfunction.
82
Interventional and Surgical Treatment of
Coarctation of the Aorta in Adults
Intervention and Peak-to-Peak Coarctation Gradient
I IIa IIb III
I IIa IIb III
Intervention for coarctation is recommended in the
following circumstances:
a) Peak-to-peak coarctation gradient greater than or equal
to 20 mm Hg.
b) Peak-to-peak coarctation gradient less than 20 mm Hg
in the presence of anatomic imaging evidence of
significant coarctation with radiological evidence of
significant collateral flow.
Choice of percutaneous catheter intervention versus
surgical repair of native discrete coarctation should be
determined by consultation with a team of ACHD
cardiologists, interventionalists, and surgeons at an
ACHD center.
83
Interventional and Surgical Treatment of
Coarctation of the Aorta in Adults
Percutaneous Catheter Intervention
I IIa IIb III
Percutaneous catheter intervention is indicated
for recurrent, discrete coarctation and a peak-topeak gradient of at least 20 mm Hg.
I IIa IIb III
Surgeons with training and expertise in CHD
should perform operations for previously repaired
coarctation and the following indications:
a) Long recoarctation segment.
b) Concomitant hypoplasia of the aortic arch.
I IIa IIb III
Stent placement for long-segment coarctation may be
considered, but the usefulness is not well established,
and the long-term efficacy and safety are unknown.
84
Intervention in Patients With
Valvular Pulmonary Stenosis
Balloon Valvotomy and Doppler Gradients
I IIa IIb III
Balloon valvotomy is recommended for asymptomatic
patients with a domed pulmonary valve and a peak
instantaneous Doppler gradient greater than 60 mm Hg
or a mean Doppler gradient greater than 40 mm Hg (in
association with less than moderate pulmonic valve
regurgitation).
I IIa IIb III
Balloon valvotomy is recommended for symptomatic
patients with a domed pulmonary valve and a peak
instantaneous Doppler gradient greater than 50 mm Hg
or a mean Doppler gradient greater than 30 mm Hg (in
association with less than moderate pulmonic
regurgitation).
85
Intervention in Patients With
Valvular Pulmonary Stenosis
Balloon Valvotomy and Doppler Gradients
I IIa IIb III
Surgical Therapy, Training, and Expertise
Surgical therapy is recommended for patients with severe
PS and an associated hypoplastic pulmonary annulus,
severe pulmonary regurgitation, subvalvular PS, or
supravalvular PS. Surgery is also preferred for most
dysplastic pulmonary valves and when there is associated
severe TR or the need for a surgical Maze procedure.
I IIa IIb III
Surgeons with training and expertise in CHD should
perform operations for the right ventricular outflow tract
(RVOT) and pulmonary valve.
86
Intervention in Patients With
Valvular Pulmonary Stenosis
Balloon Valvotomy and Doppler Gradients
I IIa IIb III
Balloon valvotomy may be reasonable in asymptomatic
patients with a dysplastic pulmonary valve and a peak
instantaneous gradient by Doppler greater than 60 mm
Hg or a mean Doppler gradient greater than 40 mm Hg.
I IIa IIb III
Balloon valvotomy may be reasonable in selected
symptomatic patients with a dysplastic pulmonary valve
and peak instantaneous gradient by Doppler greater than
50 mm Hg or a mean Doppler gradient greater than 30
mm Hg.
87
Intervention in Patients With
Valvular Pulmonary Stenosis
Balloon Valvotomy and Doppler Gradients
I IIa IIb III
Balloon valvotomy is not recommended for
asymptomatic patients with a peak instantaneous
gradient by Doppler less than 50 mm Hg in the
presence of normal cardiac output.
I IIa IIb III
Balloon valvotomy is not recommended for
symptomatic patients with PS and severe
pulmonary regurgitation.
I IIa IIb III
Balloon valvotomy is not recommended for
symptomatic patients with a peak instantaneous
gradient by Doppler less than 30 mm Hg.
88